- Email: [email protected]
Giant mediastinal neuroenteric cyst with intraspinal neurenteric cyst
European Journal of Radiology Extra 68 (2008) e59–e61
Giant mediastinal neuroenteric cyst with intraspinal neurenteric cyst Fan Zhang, Guang-ming Lu ∗ Department of Radiology, Nanjing Jinling Hospital, Clinical School of Medical College of Nanjing University, Nanjing 210002, China Received 23 April 2008; accepted 13 June 2008
Abstract Mediastinal giant neuroenteric cyst is very rare lesion. We report the imaging characteristic in a 13-year-old boy with giant and small neuroenteric cyst which had a histopathologically proven in mediastinal and cervical spinal canal, respectively. The giant neuroenteric cyst was visualized conspicuously to be in communication with the small cyst via the vertebral defect in magnetic resonance imaging (MRI). MRI could be extremely valuable in suggesting the diagnosis. © 2008 Elsevier Ireland Ltd. All rights reserved. Keywords: Neuroenteric cyst; Magnetic resonance imaging
1. Introduction Neurenteric cysts are heterotopic intraspinal cysts which result from entrapment of endodermal tissue between a segmentally split notochord [1,2]. This embryonic defect leads to a spectrum of malformations ranging from neurenteric cysts limited to the spinal cord to complex associations of spinal and extraspinal defects such as mediastinal or abdominal cysts [3,4]. In this report, we present a case of a giant intrathoracic neuroenteric cyst communicating with an intraspinal neurenteric cyst through the cervical vertebral cleft in a boy who present with symptom of spinal cord compressions. 2. Case report A 13-year-old boy presented with 10 years history of upper extremities and lower extremities numbness, especially in right side. One year before, the patient presented dyspnea after action and neck pain. Examination revealed a mild spastic tetraparesis with intact sensation, mild limitation of neck movement in all directions, difficulty in walking fast and dyspnea on small efforts as well as muscular atrophy of extremities. ∗
Corresponding author. Tel.: +86 25 80860185; fax: +86 25 80860185. E-mail addresses: mri [email protected] (F. Zhang), [email protected] (G.-m. Lu). 1571-4675/$ – see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2008.06.014
This patient underwent radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) of the thoracic part. Radiographs showed a right-sided intrathoracic mass and marked scoliosis (Fig. 1A). Coronal T2-weighted MRI revealed a giant cystic mass in right thoracic cavity and small one in C5–C6 spinal canal with extending over the same segments and marked scoliosis (Fig. 1B). Right thoracic giant cystic mass had two sacs which low sac was greater intensity than upper one on T1-weighted MRI (Fig. 1C). Right thoracic giant cystic was visualized to be in communication with the small cyst via the vertebral defect in the prevertebral, retro-aortic region (Fig. 2A). Tranaxial thoracic CT demonstrated unique density cystic mass by which trachea and esophagus was shifted forward as well as anterior spina bifida (Fig. 2B and C). A C5–C6 intradural ventral spinal cord cystic mass, which signal intensity was similar to cerebrospinal fluid, adherent to the anterior-right aspect of the spinal cord into which it had invaginated and caused severe compression of the cervical spinal cord so that spinal cord was reduced to a thin crescent posterior on axial MRI scans (Fig. 2D). The patient was submitted to a surgical treatment and complete extirpation of the cysts was performed through a right side thoracotomy. The cyst was filled with yellowish semiliquid substance, which was aspirated. Microscopic examination revealed cyst wall lined with pseudostratified cubical and columnar epithelium with thick smooth-muscle coats. There were focal
e60
F. Zhang, G.-m. Lu / European Journal of Radiology Extra 68 (2008) e59–e61
Fig. 1. Plain chest radiograph shows a large round mass in the right upper mediastinum and marked scoliosis (A). MRI showing a giant hyperintense cystic mass in right posterior upper mediastinum which have two sacs and larger sac was low-signal-intensity and small sac (arrows) was greater intensity than larger one on T1-weighted MRI and small round cystic mass (arrow) in C5–C6 spinal canal with extending over the same segments and marked scoliosis (B and C).
areas of squamous metaplasia and small nest of glandular tissue (Fig. 2E). These findings were consistent with the features of respiratory epithelium. 3. Discussion We describe a juvenile case of mediastinospinal neuroenteric cyst revealed by long-lasting chronic myelopathy and dyspnea onset on recent 1 year. Neuroenteric are very rare lesions which are posterior enteric remnants that result from incomplete separation of the notochord from the foregut in the third to fourth weeks of embryogenesis [3,4]. The presumed cause of the incomplete separation is a persistent communication or adhesion between the ectoderm of the spinal cord and the endoderm of the foregut before neural tube closure. The exact mechanism is unclear. An adhesion between the
two may cause the foregut to invaginate and pinch off, forming an enteric cyst that may demonstrate intraspinal extension which can disrupt vertebral body development and induce a sagittal cleft defect or more severe vertebral anomalies, such as scoliosis, hemivertebra and butterfly vertebrae [5]. Because of the cephalic growth of the notochord and caudal growth of the foregut, associated vertebral defects are typically superior to the mediastinal cyst [4]. In our case, above-mentioned hypotheses may explain imaging finding of our patient objectively. Neuroenteric cyst can be extradural or intradural, and occasionally intramedullary. Intrathoracic cysts can coexist with neuroenteric cyst [6,7]. About 90% of intrathoracic neurenteric cysts occur in the right posterior mediastinum, superior to the carina, and separate from the esophagus. The clinical presentation are associated with these lesions derive mainly from mass
Fig. 2. T2-weighted sagittal image shows an iso-intense with CSF, intradural extramedullary ventral cystic mass extending to the extraspinal space, the posterior mediastinum, through a defect in the vertebral body (arrow) (A). CT shows a defect in the body of the thoracic spine: anterior spina bifida (arrow) and the giant unenhanced isodensity cystic mass in the right thoracic cavity (arrow) by which trachea and esophagus are shifted forward (B and C). T2-weighted axial images show high-signal-intensity cystic lesions (arrow) adherent to the anterior-right aspect of the spinal cord into which it had invaginated and compress the cervical spinal cord (D). Photomicrograph of the neurenteric cyst wall lined by pseudostratified cuboidal and columnar epithelium with underlying focus of glandular tissue (hematoxylin-eosin; original magnification 100×) (E).
F. Zhang, G.-m. Lu / European Journal of Radiology Extra 68 (2008) e59–e61
effect. Pressure on the spinal cord may produce weakness, pain, extremities numbness, muscular atrophy and myelopathy, resulting in ataxia and incontinence. Furthermore, intrathoracic cysts can become large enough to cause cardiac or respiratory problems [8], as in our patient. Neuroenteric cyst is also referred to as endodermal, enterogenous, enteric, foregut, epithelial, bronchogenic, respiratory, and neuroepithelial cysts. This terminology obviously differs because of the various disciplines involved that view the lesion from different perspectives. Neckrysh et al. [9] thought this reflects the confusion with understanding the histogenesis of these lesions and underestimation of the true incidence of neuroenteric cysts as well as quite misleading microscopic appearance. Kim et al. [10] reviewed some case of neuroenteric cysts, which the histopathological appearances are similar to that of respiratory epithelium and is consistent with an endodermic origin, as in our patient. Neuroenteric cyst morphologic features, location, and imaging characteristics are very helpful in suggesting the correct diagnosis and differential diagnosis from other intrathoracic cysts of childhood such as bronchogenic cysts. Successful treatment can be expected when these combined malformations are diagnosed accurately at onset. Of the neuroimaging modalities, MRI has replaced other techniques to better characterize the neuroenteric cyst [11]. MRI defines the anatomic features of the lesions. In most instances, sagittal, axial, and coronal T1weighted and T2-weighted images will suffice to reliably depict the characteristic the neuroenteric cyst and relative malformation [12]. In conclusion, although neuroenteric cyst is a congenital lesion, it is usually not detected until the neurological symptoms
e61
have become obvious. Clinical suspicion and imaging examination are important for early diagnosis and treatment. References [1] Agnoli AL, Albrecht L, Schonmayr R. Enterogenous intraspinal cysts. J Neurosurg 1984;61:834–40. [2] Agrawal D, Suri A, Mahapatra AK, et al. Intramedullary neurenteric cyst presenting as infantile paraplegia: a case and review. Pediatr Neurosurg 2002;37:93–6. [3] Strollo DC, Rosado-de-Christenson ML, Jett JR. Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastina. Chest 1997;112:1344–52. [4] Kirwan WO, Walbaum PR, McCormack RJM. Cystic intrathoracic derivatives of the foregut and their complications. Thorax 1973;28:424–8. [5] Arnold HM, Vincent CT. Spinal neurenteric cysts in the magnetic resonance imaging era. J Neurosurg 2006;58:97–105. [6] Brianp W, John DU, Joseph GA, et al. Primary tumors of the mediastinum. J Surg Oncol 1999;70:95–9. [7] Lippman CR, Arginteanu M, Purohit D, et al. Intramedullary neurenteric cysts of the spine. Case report and review of the literature. J Neurosurg 2001;94:305–9. [8] Corbett CW, Craig TA, Russell WJ, et al. Fetal neurenteric cyst causing hydrops: case report and review of the literature. Prenat Diagn 1999;19:118–21. [9] Neckrysh S, Valyi-Naqv T, Charbel FT. Neuroenteric cyst of the anterior cranial fossa: case report and review of the literature. Surg Neurol 2006;65:174–7. [10] Kim CY, Wang KC, Choe G, et al. Neurenteric cyst: its various presentations. Childs Nerv Syst 1999;15:333–41. [11] Aoki S, Machida T, Sasaki Y, et al. Enterogenous cyst of cervical spine: clinical and radiological aspect (including CT and MRI). Neuroradiology 1987;29:291–3. [12] Brooks BS, Duvall ER, Gammal TE, et al. Neuroimaging features of neurenteric cysts: analysis of nine cases and review of the literature. AJNR Am J Neuroradiol 1993;14:735–46.
Giant mediastinal neuroenteric cyst with intraspinal neurenteric cyst Fan Zhang, Guang-ming Lu ∗ Department of Radiology, Nanjing Jinling Hospital, Clinical School of Medical College of Nanjing University, Nanjing 210002, China Received 23 April 2008; accepted 13 June 2008
Abstract Mediastinal giant neuroenteric cyst is very rare lesion. We report the imaging characteristic in a 13-year-old boy with giant and small neuroenteric cyst which had a histopathologically proven in mediastinal and cervical spinal canal, respectively. The giant neuroenteric cyst was visualized conspicuously to be in communication with the small cyst via the vertebral defect in magnetic resonance imaging (MRI). MRI could be extremely valuable in suggesting the diagnosis. © 2008 Elsevier Ireland Ltd. All rights reserved. Keywords: Neuroenteric cyst; Magnetic resonance imaging
1. Introduction Neurenteric cysts are heterotopic intraspinal cysts which result from entrapment of endodermal tissue between a segmentally split notochord [1,2]. This embryonic defect leads to a spectrum of malformations ranging from neurenteric cysts limited to the spinal cord to complex associations of spinal and extraspinal defects such as mediastinal or abdominal cysts [3,4]. In this report, we present a case of a giant intrathoracic neuroenteric cyst communicating with an intraspinal neurenteric cyst through the cervical vertebral cleft in a boy who present with symptom of spinal cord compressions. 2. Case report A 13-year-old boy presented with 10 years history of upper extremities and lower extremities numbness, especially in right side. One year before, the patient presented dyspnea after action and neck pain. Examination revealed a mild spastic tetraparesis with intact sensation, mild limitation of neck movement in all directions, difficulty in walking fast and dyspnea on small efforts as well as muscular atrophy of extremities. ∗
Corresponding author. Tel.: +86 25 80860185; fax: +86 25 80860185. E-mail addresses: mri [email protected] (F. Zhang), [email protected] (G.-m. Lu). 1571-4675/$ – see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2008.06.014
This patient underwent radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) of the thoracic part. Radiographs showed a right-sided intrathoracic mass and marked scoliosis (Fig. 1A). Coronal T2-weighted MRI revealed a giant cystic mass in right thoracic cavity and small one in C5–C6 spinal canal with extending over the same segments and marked scoliosis (Fig. 1B). Right thoracic giant cystic mass had two sacs which low sac was greater intensity than upper one on T1-weighted MRI (Fig. 1C). Right thoracic giant cystic was visualized to be in communication with the small cyst via the vertebral defect in the prevertebral, retro-aortic region (Fig. 2A). Tranaxial thoracic CT demonstrated unique density cystic mass by which trachea and esophagus was shifted forward as well as anterior spina bifida (Fig. 2B and C). A C5–C6 intradural ventral spinal cord cystic mass, which signal intensity was similar to cerebrospinal fluid, adherent to the anterior-right aspect of the spinal cord into which it had invaginated and caused severe compression of the cervical spinal cord so that spinal cord was reduced to a thin crescent posterior on axial MRI scans (Fig. 2D). The patient was submitted to a surgical treatment and complete extirpation of the cysts was performed through a right side thoracotomy. The cyst was filled with yellowish semiliquid substance, which was aspirated. Microscopic examination revealed cyst wall lined with pseudostratified cubical and columnar epithelium with thick smooth-muscle coats. There were focal
e60
F. Zhang, G.-m. Lu / European Journal of Radiology Extra 68 (2008) e59–e61
Fig. 1. Plain chest radiograph shows a large round mass in the right upper mediastinum and marked scoliosis (A). MRI showing a giant hyperintense cystic mass in right posterior upper mediastinum which have two sacs and larger sac was low-signal-intensity and small sac (arrows) was greater intensity than larger one on T1-weighted MRI and small round cystic mass (arrow) in C5–C6 spinal canal with extending over the same segments and marked scoliosis (B and C).
areas of squamous metaplasia and small nest of glandular tissue (Fig. 2E). These findings were consistent with the features of respiratory epithelium. 3. Discussion We describe a juvenile case of mediastinospinal neuroenteric cyst revealed by long-lasting chronic myelopathy and dyspnea onset on recent 1 year. Neuroenteric are very rare lesions which are posterior enteric remnants that result from incomplete separation of the notochord from the foregut in the third to fourth weeks of embryogenesis [3,4]. The presumed cause of the incomplete separation is a persistent communication or adhesion between the ectoderm of the spinal cord and the endoderm of the foregut before neural tube closure. The exact mechanism is unclear. An adhesion between the
two may cause the foregut to invaginate and pinch off, forming an enteric cyst that may demonstrate intraspinal extension which can disrupt vertebral body development and induce a sagittal cleft defect or more severe vertebral anomalies, such as scoliosis, hemivertebra and butterfly vertebrae [5]. Because of the cephalic growth of the notochord and caudal growth of the foregut, associated vertebral defects are typically superior to the mediastinal cyst [4]. In our case, above-mentioned hypotheses may explain imaging finding of our patient objectively. Neuroenteric cyst can be extradural or intradural, and occasionally intramedullary. Intrathoracic cysts can coexist with neuroenteric cyst [6,7]. About 90% of intrathoracic neurenteric cysts occur in the right posterior mediastinum, superior to the carina, and separate from the esophagus. The clinical presentation are associated with these lesions derive mainly from mass
Fig. 2. T2-weighted sagittal image shows an iso-intense with CSF, intradural extramedullary ventral cystic mass extending to the extraspinal space, the posterior mediastinum, through a defect in the vertebral body (arrow) (A). CT shows a defect in the body of the thoracic spine: anterior spina bifida (arrow) and the giant unenhanced isodensity cystic mass in the right thoracic cavity (arrow) by which trachea and esophagus are shifted forward (B and C). T2-weighted axial images show high-signal-intensity cystic lesions (arrow) adherent to the anterior-right aspect of the spinal cord into which it had invaginated and compress the cervical spinal cord (D). Photomicrograph of the neurenteric cyst wall lined by pseudostratified cuboidal and columnar epithelium with underlying focus of glandular tissue (hematoxylin-eosin; original magnification 100×) (E).
F. Zhang, G.-m. Lu / European Journal of Radiology Extra 68 (2008) e59–e61
effect. Pressure on the spinal cord may produce weakness, pain, extremities numbness, muscular atrophy and myelopathy, resulting in ataxia and incontinence. Furthermore, intrathoracic cysts can become large enough to cause cardiac or respiratory problems [8], as in our patient. Neuroenteric cyst is also referred to as endodermal, enterogenous, enteric, foregut, epithelial, bronchogenic, respiratory, and neuroepithelial cysts. This terminology obviously differs because of the various disciplines involved that view the lesion from different perspectives. Neckrysh et al. [9] thought this reflects the confusion with understanding the histogenesis of these lesions and underestimation of the true incidence of neuroenteric cysts as well as quite misleading microscopic appearance. Kim et al. [10] reviewed some case of neuroenteric cysts, which the histopathological appearances are similar to that of respiratory epithelium and is consistent with an endodermic origin, as in our patient. Neuroenteric cyst morphologic features, location, and imaging characteristics are very helpful in suggesting the correct diagnosis and differential diagnosis from other intrathoracic cysts of childhood such as bronchogenic cysts. Successful treatment can be expected when these combined malformations are diagnosed accurately at onset. Of the neuroimaging modalities, MRI has replaced other techniques to better characterize the neuroenteric cyst [11]. MRI defines the anatomic features of the lesions. In most instances, sagittal, axial, and coronal T1weighted and T2-weighted images will suffice to reliably depict the characteristic the neuroenteric cyst and relative malformation [12]. In conclusion, although neuroenteric cyst is a congenital lesion, it is usually not detected until the neurological symptoms
e61
have become obvious. Clinical suspicion and imaging examination are important for early diagnosis and treatment. References [1] Agnoli AL, Albrecht L, Schonmayr R. Enterogenous intraspinal cysts. J Neurosurg 1984;61:834–40. [2] Agrawal D, Suri A, Mahapatra AK, et al. Intramedullary neurenteric cyst presenting as infantile paraplegia: a case and review. Pediatr Neurosurg 2002;37:93–6. [3] Strollo DC, Rosado-de-Christenson ML, Jett JR. Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastina. Chest 1997;112:1344–52. [4] Kirwan WO, Walbaum PR, McCormack RJM. Cystic intrathoracic derivatives of the foregut and their complications. Thorax 1973;28:424–8. [5] Arnold HM, Vincent CT. Spinal neurenteric cysts in the magnetic resonance imaging era. J Neurosurg 2006;58:97–105. [6] Brianp W, John DU, Joseph GA, et al. Primary tumors of the mediastinum. J Surg Oncol 1999;70:95–9. [7] Lippman CR, Arginteanu M, Purohit D, et al. Intramedullary neurenteric cysts of the spine. Case report and review of the literature. J Neurosurg 2001;94:305–9. [8] Corbett CW, Craig TA, Russell WJ, et al. Fetal neurenteric cyst causing hydrops: case report and review of the literature. Prenat Diagn 1999;19:118–21. [9] Neckrysh S, Valyi-Naqv T, Charbel FT. Neuroenteric cyst of the anterior cranial fossa: case report and review of the literature. Surg Neurol 2006;65:174–7. [10] Kim CY, Wang KC, Choe G, et al. Neurenteric cyst: its various presentations. Childs Nerv Syst 1999;15:333–41. [11] Aoki S, Machida T, Sasaki Y, et al. Enterogenous cyst of cervical spine: clinical and radiological aspect (including CT and MRI). Neuroradiology 1987;29:291–3. [12] Brooks BS, Duvall ER, Gammal TE, et al. Neuroimaging features of neurenteric cysts: analysis of nine cases and review of the literature. AJNR Am J Neuroradiol 1993;14:735–46.